We studied granulocyte colony-stimulating factor (G-CSF) binding sites on neutrophils from patients with severe congenital neutropenia (SCN; Kostmann-syndrome) and cyclic neutropenia (CN) during treatment with recombinant human (rh) G-CSF. G-CSF receptor expression was measured by scatchard analysis. Neutrophils from six healthy controls expressed between 480 and 1,210 binding sites per cell, w...

Background: Late onset neutropenia (neutropenia after 3 weeks of life) may be a physiological condition without need to prescribe antibiotics, G-CSF, or IVIG. We aimed to determine the association between sepsis and late onset neutropenia in very low birth weight (<1500 gm) infants.&nbsp; Methods: This study was a cross-sectional prospective study in VLBW infants that were admitted in Mahdieh ...

The phagocytic, bactericidal, and metabolic activities of monocytes from patients with congenital neutropenia were not significantly different than the activities of monocytes from patients with cyclic neutropenia or chronic infections. However, significant differences in metabolic, phagocytic, and bactericidal functions were found between monocytes from any source and normal neutrophils. Durin...

Clericuzio-type poikiloderma with neutropenia (PN) is characterized by poikiloderma, non-cyclic neutropenia, recurrent sinopulmonary infections, pachyonychia, and palmo-plantar hyperkeratosis. Mutations in the C16orf57 gene, which is located on chromosome 16q13, have been identified as the cause of PN. PN was first described by Clericuzio in Navajo Indians. Herein, we reported the clinical pres...

Severe congenital neutropenia (SCN) and cyclic neutropenia (CyN) are sporadic or inherited hematologic disorders of myelopoiesis. Heterozygous mutations in the gene encoding neutrophil elastase (ELA2) have been reported in both diseases. We used an inducible system to express a panel of ELA2 mutations and found for almost all mutants disruption of intracellular neutrophil elastase (HNE) protein...

Cyclic neutropenia is a rare disease that occurs both in humans and gray collie dogs and is characterized by recurrent severe neutropenia leading to bacterial infections and shortened life expectancy. Daily injections of recombinant granulocyte colony-stimulating factor (rG-CSF) are effective in shortening the period of severe neutropenia and reducing infections. After demonstrating that rG-CSF...

Severe congenital neutropenia (SCN) is characterised by a differentiation block in the bone marrow and low neutrophil numbers in the peripheral blood, which correlates with increased risk of bacterial infections. Several underlying gene defects have been identified in SCN patients. Mutations in the neutrophil elastase (ELANE) gene are frequently found in SCN and cyclic neutropenia. Both misloca...

A highly regio- and stereo-selective synthesis of cyclic β-D-glucosides 3 via Pd(0)-catalyzed coupling cyclization of allenyl β-D-glucoside 1 and organic iodides in 20-38% yields is reported. After the deacetylation of 3, we obtained cyclic β-D-glucoses 4 in 90-97% yields, which may have SGLT2 inhibition activities.

Cyclic Neutropenia (CN) is characterized by recurrent infections, fever, oral ulcerations, and severe periodontitis as result of the reduced host defences. The previous studies have established the effectiveness of recombinant granulocyte colony-stimulating factor (GCSF) to increase the number and the function of neutrophils in the peripheral blood in this disease. In a 20-year-old Caucasian fe...